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| {{Infobox_Disease |
| | __NOTOC__ |
| Name = {{PAGENAME}} |
| | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
| Image = ARF mitral valve.jpg|
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| Caption = Rheumatic Mitral Valvulitis: Gross; an excellent example of acute rheumatic fever lesion along line of closure of mitral valve <br> <small> [http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small>|
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| DiseasesDB = 11487 |
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| ICD10 = {{ICD10|I|00||i|00}}-{{ICD10|I|02||i|00}} |
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| ICD9 = {{ICD9|390}}–{{ICD9|392}} |
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| ICDO = |
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| OMIM = |
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| MedlinePlus = 003940 |
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| eMedicineSubj = med |
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| eMedicineTopic = 3435 |
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| eMedicine_mult = {{eMedicine2|med|2922}} {{eMedicine2|emerg|509}} {{eMedicine2|ped|2006}} |
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| MeshID = D012213 |
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| }}
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| {{SI}}
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| {{WikiDoc Cardiology Network Infobox}}
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| '''Editor-in-Chief:''' Lance Christiansen, D.O.
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| '''Associate Editor:''' {{CZ}}
| | {{Rheumatic fever}} |
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| {{Editor Help}} | | {{CMG}}; Lance Christiansen, D.O.; {{AE}} {{CZ}}; {{VK}}; {{AG}} |
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| ==Overview==
| | '''''Synonyms and Keywords:''''' RF; Rheumatic heart disease; RHD; Acute rheumatic fever; Chronic rheumatic fever; Rheumatic carditis |
| '''Rheumatic fever''' The pathological, high-level, systemic, autoimmunological, inflammatory reaction, which is a ''sequela'' to an infection from a virulent strain of Streptococcus pyogenes bacteria, in an individual who has had prior, proper sensitization to Streptococcus pyogenes' autoantigens from prior infections by Streptococcus pyogenes, is termed rheumatic fever. The systemic signs and symptoms of rheumatic fever are caused by an attack on an individual's tissues by autoantibodies that are self-developed during an immunological response to Streptococcus pyogenes autoantigens. The disease state of rheumatic fever is due to an individual's autoimmunological response to the autoantigens of Streptococcus pyogenes during an infection and not from the Streptococcus pyogenes infection itself. | |
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| ==General information== | | ==[[Rheumatic fever overview|Overview]]== |
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| '''Rheumatic fever''' is common worldwide and responsible for many cases of damaged [[heart valve]]s. In the Western countries, it became fairly rare since the 1960s, probably due to widespread use of antibiotics to treat [[streptococcus]] infections. While it is far less common in the United States since the beginning of the 20th century, there have been a few outbreaks since the 1980s. Although the disease seldom occurs, it is serious and has a mortality of 2–5%.
| | ==[[Rheumatic fever historical perspective|Historical Perspective]]== |
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| Rheumatic fever primarily affects children between ages 6 and 15 years and occurs approximately 20 days after strep throat or [[scarlet fever]]. In up to a third of cases, the underlying strep infection may not have caused any symptoms. | |
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| The rate of development of rheumatic fever in individuals with untreated strep infection is estimated to be 3%. The rate of development is far lower in individuals who have received antibiotic treatment. Persons who have suffered a case of rheumatic fever have a tendency to develop flare-ups with repeated strep infections.
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| The recurrence of rheumatic fever is relatively common in the absence of maintenance of low dose antibiotics, especially during the first three to five years after the first episode. Heart complications may be long-term and severe, particularly if valves are involved.
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| ==Diagnosis: modified Jones criteria== | | ==[[Rheumatic fever classification|Classification]]== |
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| T. Duckett Jones, MD, first published these criteria in 1944.<ref>Jones TD. The diagnosis of rheumatic fever. ''[[Journal of the American Medical Association|JAMA]]''. 1944; 126:481–484</ref> They have been periodically revised by the [[American Heart Association]] in collaboration with other groups.<ref>[http://circ.ahajournals.org/cgi/content/full/106/19/2521?ck=nck Ferrieri P. Proceedings of the Jones criteria workshop]. ''Circulation'' 2002; 106: 2521–23</ref> '''Two major criteria, or one major and two minor criteria''', when there is also evidence of a previous strep infection, support the diagnosis of rheumatic fever.<ref>{{cite web |url=http://www.emedicine.com/emerg/topic509.htm |title=eMedicine — Rheumatic Fever | author = Steven J Parrillo, DO, FACOEP, FACEP|format= |work=}}</ref><ref>{{cite journal |author= |title=Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association |journal=JAMA |volume=268 |issue=15 |pages=2069-73 |year=1992 |pmid=1404745 |doi=}}</ref>
| | ==[[Rheumatic fever pathophysiology|Pathophysiology]]== |
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| ===Major criteria===
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| *'''Carditis:''' inflammation of the heart muscle which can manifest as [[congestive heart failure]] with shortness of breath, [[pericarditis]] with a rub, or a new [[heart murmur]].
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| *'''[[Arthritis|Migratory polyarthritis]]:''' a temporary migrating inflammation of the large joints, usually starting in the legs and migrating upwards.
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| *'''[[Sydenham's chorea]] (St. Vitus' dance):''' a characteristic series of rapid movements without purpose of the face and arms. This can occur very late in the disease.
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| *'''[[Erythema marginatum]]:''' a long lasting rash that begins on the trunk or arms as [[macule]]s and spread outward to form a snakelike ring while clearing in the middle. This rash never starts on the face and is made worse with heat.
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| *'''Subcutaneous nodules (a form of [[Aschoff bodies]]):''' painless, firm collections of collagen fibers on the back of the wrist, the outside elbow, and the front of the knees. These now occur infrequently.
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| ===Minor criteria=== | | ==[[Rheumatic fever causes|Causes]]== |
| *'''[[Fever]]''': temperature elevation
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| *'''[[Arthralgia]]:''' Joint pain without swelling
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| *'''Laboratory abnormalities:''' increased [[Erythrocyte sedimentation rate]], increased [[C reactive protein]], [[leukocytosis]]
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| *'''[[Electrocardiogram]]''' abnormalities: a prolonged PR interval
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| *'''Evidence of Group A Strep infection:''' positive culture for Group A Strep, elevated or rising [[Antistreptolysin O titre]]
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| *'''Previous rheumatic fever or inactive heart disease'''
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| ===Other signs and symptoms=== | | ==[[Rheumatic fever differential diagnosis|Differentiating Rheumatic Fever from other Diseases]]== |
| *[[Abdominal pain]]
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| *[[Nosebleed]]s
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| ==Pathophysiology== | | ==[[Rheumatic fever epidemiology and demographics|Epidemiology and Demographics]]== |
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| [[Rheumatic fever]] is a systemic disease affecting the peri-arteriolar connective tissue and can occur after an untreated Group A streptococcal pharyngeal infection. It is believed to be caused by antibody [[cross-reactivity]]. This cross-reactivity is a Type II hypersensitivity reaction and is termed ''molecular mimicry.'' | | ==[[Rheumatic fever risk factors|Risk Factors]]== |
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| Usually, self reactive B cells remain anergic in the periphery without T cell co-stimulation. During a Strep. infection activated antigen presenting cells such as macrophages present the bacterial antigen to helper T cells. Helper T cells subsequently activate B cells and induce the production of antibodies against the cell wall of Streptococcus. However the antibodies may also react against the myocardium and joints<ref>Abbas and Lechtman. m'''Basic Immunology: Functions and Disorders of the Immune System'''. Elsevier Inc. 2004.</ref>, producing the symptoms of Rheumatic fever.
| | ==[[Rheumatic fever screening|Screening]]== |
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| Group A ''[[streptococcus pyogenes]]'' has a [[cell wall]] composed of branched [[polymers]] which sometimes contain "''M proteins''" that are highly [[antigenic]]. The antibodies which the immune system generates against the "''M proteins''" may cross react with cardiac myofiber [[sarcolemma]] and smooth muscle cells of arteries, inducing [[cytokine]] release and tissue destruction. This inflammation occurs through direct attachment of complement and Fc receptor-mediated recruitment of neutrophils and macrophages. Characteristic Aschoff bodies, composed of swollen eosinophilic collagen surrounded by lymphocytes and macrophages can be seen on light microscopy. The larger macrophages may become Aschoff giant cells. Acute rheumatic valvular lesions may also involve a [[cell-mediated immunity]] reaction as these lesions predominantly contain [[T-helper]] cells and [[macrophages]].<ref>Kumar et al. '''Robbins and Cotran Pathologic Basis of Disease'''. Elsevier Inc. 2005</ref>
| | ==[[Rheumatic fever natural history, complications, and prognosis|Natural History, Complications, and Prognosis]]== |
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| In acute RF, these lesions can be found in any layer of the heart and is hence called pancarditis. The inflammation may cause a serofibrinous pericardial exudates described as “bread-and-butter” pericarditis, which usually resolves without sequelae. Involvement of the endocardium typically results in fibrinoid necrosis and verrucae formation along the lines of closure of the left-sided heart valves. Warty projections arise from the deposition, while subendothelial lesions may induce irregular thickenings called [[MacCallum plaques]].
| | ==Diagnosis== |
| | | [[Jones criteria|Jones Criteria]] | [[Rheumatic fever history and symptoms|History and Symptoms]] | [[Rheumatic fever physical examination|Physical Examination]] | [[Rheumatic fever laboratory findings|Laboratory Findings]] | [[Rheumatic fever electrocardiogram|Electrocardiogram]] | [[Rheumatic fever chest x ray|Chest X Ray]] | [[Rheumatic fever echocardiography or ultrasound|Echocardiography]] |
| Chronic rheumatic heart disease is characterized by repeated inflammation with fibrinous resolution. The cardinal anatomic changes of the valve include leaflet thickening, commissural fusion and shortening and thickening of the tendinous cords. RHD cause 99% of mitral stenosis often resulting in a “fish mouth” gross appearance.<ref>{{cite web |url=http://www.robbinspathology.com/ |title=Robbins & Cotran Pathologic Basis of Disease |format= |work=}}</ref>
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| ==Treatment== | | ==Treatment== |
| | [[Rheumatic fever medical therapy|Medical therapy]] | [[Rheumatic fever primary prevention|Primary Prevention]] | [[Rheumatic fever secondary prevention|Secondary Prevention]] |
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| The management of acute rheumatic fever is geared toward the reduction of inflammation with [[anti-inflammatory medication]]s such as [[aspirin]] or [[corticosteroid]]s. Individuals with positive cultures for strep throat should also be treated with [[antibiotic]]s. Another important cornerstone in treating rheumatic fever includes the continuous use of low dose antibiotics (such as [[penicillin]], [[sulfadiazine]], or [[erythromycin]]) to prevent recurrence.
| | ==Case Studies== |
| | | [[Acute rheumatic fever case study one|Case #1]] |
| ===Infection=== | |
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| Patients with positive cultures for ''streptococcus pyogenes'' should be treated with penicillin as long as [[allergy]] is not present. This treatment will not alter the course of the acute disease.
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| ===Inflammation===
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| Patients with significant symptoms may require [[corticosteroids]]. [[Salicylates]] are useful for pain.
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| ===Heart failure===
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| Some patients develop significant [[carditis]] which manifests as [[congestive heart failure]]. This requires the usual treatment for heart failure: [[diuretics]] and [[digoxin]]. Unlike normal heart failure, rheumatic heart failure responds well to [[corticosteroids]].
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| ==Prevention==
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| Prevention of recurrence is achieved by eradicating the acute infection and [[prophylaxis]] with antibiotics. The [[American Heart Association]] recommends daily or monthly prophylaxis continue long-term, perhaps for life.<ref name="aha">{{cite web|title=Rheumatic Heart Disease/Rheumatic Fever|url=http://www.americanheart.org/presenter.jhtml?identifier=4709|publisher=American Heart Association|accessdate=2008-02-17}}</ref>
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| Primary care physicians, nurses also have a role in prevention, primarily in screening school-aged children for sore throats that may be caused by Group A streptococci(especially Group A β Hemolytic Streptococcus pyogenes).
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| ==Pathological Findings==
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| [http://www.peir.net Images courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology]
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| <gallery>
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| Image:Rheumatic fever 001.jpg|Aortic Stenosis (Tricuspid aorta): Gross, good example of aortic stenosis due to rheumatic fever
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| Image:Rheumatic fever 002.jpg|Gross, an excellent example of mitral scarring due to rheumatic fever (healing phase of an infectious lesion).
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| Image:Rheumatic mitral valvulitis.jpg|Rheumatic mitral valvulitis: Gross, an excellent example of fibrosis, chorda thickening and shortening has thrombus around the large left atrium
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| </gallery>
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| <gallery>
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| Image:Rheumatic fever 003.jpg|Mitral valve: acute rheumatic fever
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| Image:Rheumatic fever 004.jpg|Aschoff bodies in rheumatic heart disease
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| </gallery>
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| ==References==
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| {{Reflist}}
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| ==External links==
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| * [http://heartcenter.seattlechildrens.org/conditions_treated/rheumatic_heart_disease.asp Rheumatic fever information] from Seattle Children's Hospital Heart Center
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| {{Electrocardiography}}
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| {{Circulatory system pathology}} | | {{Circulatory system pathology}} |
| {{Bacterial diseases}} | | {{Bacterial diseases}} |
| {{Hypersensitivity and autoimmune diseases}} | | {{Hypersensitivity and autoimmune diseases}} |
| {{SIB}}
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| [[zh-min-nan:Hong-sip-jia̍t]]
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| [[de:Rheumatisches Fieber]]
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| [[fr:Rhumatisme articulaire aigu]]
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| [[it:Febbre reumatica]]
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| [[nl:Acuut reuma]]
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| [[ja:リウマチ熱]]
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| [[pl:Gorączka reumatyczna]]
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| [[sr:Реуматска грозница]]
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| [[Category:Cardiology]] | | [[Category:Cardiology]] |
| | [[Category:Rheumatology]] |
| [[Category:Bacterial diseases]] | | [[Category:Bacterial diseases]] |
| [[Category:Rheumatology]]
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